Kral Andrej, Kronenberger William G, Pisoni David B, O'Donoghue Gerard M
Institute of AudioNeuroTechnology and Department of Experimental Otology, ENT Clinics, Medical University Hannover, Hannover, Germany; School of Behavioural and Brain Sciences, The University of Texas at Dallas, Dallas, TX, USA.
Department of Psychiatry, and DeVault Otologic Research Laboratory, Department of Otolaryngology: Head and Neck Surgery, Indiana University School of Medicine, Indianapolis, IN, USA; Department of Psychological and Brain Sciences, Indiana University, Indianapolis, IN, USA.
Lancet Neurol. 2016 May;15(6):610-21. doi: 10.1016/S1474-4422(16)00034-X. Epub 2016 Mar 12.
Progress in biomedical technology (cochlear, vestibular, and retinal implants) has led to remarkable success in neurosensory restoration, particularly in the auditory system. However, outcomes vary considerably, even after accounting for comorbidity-for example, after cochlear implantation, some deaf children develop spoken language skills approaching those of their hearing peers, whereas other children fail to do so. Here, we review evidence that auditory deprivation has widespread effects on brain development, affecting the capacity to process information beyond the auditory system. After sensory loss and deafness, the brain's effective connectivity is altered within the auditory system, between sensory systems, and between the auditory system and centres serving higher order neurocognitive functions. As a result, congenital sensory loss could be thought of as a connectome disease, with interindividual variability in the brain's adaptation to sensory loss underpinning much of the observed variation in outcome of cochlear implantation. Different executive functions, sequential processing, and concept formation are at particular risk in deaf children. A battery of clinical tests can allow early identification of neurocognitive risk factors. Intervention strategies that address these impairments with a personalised approach, taking interindividual variations into account, will further improve outcomes.
生物医学技术（人工耳蜗、前庭植入物和视网膜植入物）的进步在神经感觉恢复方面取得了显著成功，尤其是在听觉系统。然而，即使考虑到合并症，结果仍有很大差异——例如，人工耳蜗植入后，一些失聪儿童发展出接近其听力正常同龄人水平的口语技能，而其他儿童则未能如此。在此，我们回顾了证据表明听觉剥夺对大脑发育有广泛影响，影响了超出听觉系统的信息处理能力。在感觉丧失和失聪后，大脑的有效连接性在听觉系统内、感觉系统之间以及听觉系统与服务于高级神经认知功能的中枢之间发生改变。因此，先天性感觉丧失可被视为一种连接组疾病，大脑对感觉丧失的个体适应性差异是人工耳蜗植入结果中观察到的大部分差异的基础。不同的执行功能、序列处理和概念形成在失聪儿童中尤其危险。一系列临床测试可以早期识别神经认知风险因素。采用个性化方法、考虑个体差异来解决这些损伤的干预策略将进一步改善结果。
